tbuckley/cabot-search huggingface dataset:
P-017: POINT-OF-CARE DIAGNOSTIC FOR QUANTIFICATION OF FOETAL HAEMOGLOBIN (HB F) LEVELS IN MONITORING HYDROXYUREA THERAPY FOR CHILDREN WITH SICKLE CELL DISEASE (SCD) IN GHANA
Authors: SEGBEFIA C., DEI-ADOMAKOH Y., MENSAH E., THOTA P., ODAME I.
Journal: HemaSphere (2022)

Purpose: Sickle cell disease (SCD) potentially causes numerous acute and chronic complications across the lifespan. The majority of patients with SCD live in sub-Saharan Africa and 1 in 50 Ghanaian newborns are affected. Foetal hemoglobin (Hb F) level is a known modulator of the severity of SCD. High levels of Hb F inhibit sickling and correlate with reduced disease morbidity and mortality. Hb F monitoring is also used to assess adherence and response to disease modifying (hydroxyurea) therapy. Standard hemoglobin electrophoretic techniques are unable to quantify Hb F. In resource-limited settings like Ghana, the use of high performance liquid chromatography (HPLC) for Hb F quantification is often challenging due to high cost and unavailability of laboratory equipment and skilled technicians. We report on the performance of a low-cost, point-of-care, microchip based cellulose acetate electrophoresis “Gazelle” compared to HPLC for Hb F quantification in Ghanaian children with SCD. GazelleTM is a fast (<8 minutes), easy-to-use test which can be performed by minimally trained personnel using only a finger-prick volume of blood. Materials and methods: The study was conducted at the paediatric SCD Clinic in Korle Bu Teaching Hospital, Accra, Ghana. Children (age ≥ 1 year to 16 years) known to have sickle cell disease who are on hydroxyurea therapy, and followed at the paediatric sickle cell clinic were enrolled in the study. The study is still ongoing. Results: A total of 110 children were included in the analysis. Gazelle showed a correlation of 0.93 when compared to HPLC (Figure 1). Conclusion: Haemoglobin variants are quantified by Gazelle offering the ability to monitor disease modifying therapy in SCD such as hydroxyurea, where quantification of HbF levels is the key to assessing treatment adherence and determining response to therapy. Gazelle has the potential to be utilized as a POC test for quantification of foetal haemoglobin (Hb F) levels in monitoring hydroxyurea therapy for children with SCD.Hb Quantification Performance of Gazelle compared to HPLC C. SEGBEFIA declares a conflict of interest: Consultancy, Expert: Novartis - Advisory Board / Steering Committee Member Global Blood Therapeutics - DSMB Member P. THOTA declares a conflict of interest: Other: Employee of Hemex Health
P-086: POINT-OF-CARE SCREENING FOR SICKLE CELL DISEASE IN RURAL AFRICAN COMMUNITY HEALTHCARE CENTERS
Authors: JAJA C., IBEMERE S., VICK L., EDEM-HOTAH J., GIBSON R.
Journal: HemaSphere (2022)

Purpose: Over 200 000 babies are born with sickle cell disease (SCD) annually in sub-Saharan Africa. In many African nations, however, 50% to 90% of children with SCD will not survive until their fifth birthday and will die due to susceptibility to malaria, sepsis, anemia, and infection without being diagnosed with SCD. The incidence of SCD and trait in newborns in Sierra Leone is unknown. Early diagnosis of SCD and treatment can greatly improve patient outcomes. The purpose of this study was to assess the feasibility of a Nurse Champion sickle cell disease point of care testing (SCD-POCT) model in community healthcare centers using a novel microtechnology diagnostic device. Materials and methods: This cross-sectional study was conducted at a regional government hospital in a rural district in Sierra Leone. Immediate, free screening was offered to newborns over 24 hours old delivered at the maternity ward. We used a validated, low-cost, point-of-care, lateral flow immunoassay (SickleSCAN®) device to screen for SCD and trait. The screening tests were conducted by 12 nurse-midwives. A brief questionnaire was used to record the test evaluators’ experience with the device. Results: Fifty-two (30 males/22 females) newborns screened within 24-48 hours of birth over four weeks. Two newborns were identified with sickle cell disease (HbSS). The test identified 13 newborns with sickle cell trait (HbAS). Thirty-seven newborns had the normal HbAA genotype. The heterozygous HbSC genotype was not observed in the study cohort. 12 nurse-midwives evaluators rated the acceptability and feasibility of screening for SCD at the bedside as very good. None of the evaluators deemed the device to be inappropriate for SCD screening. Conclusion: Our study demonstrates a practical method of instituting SCD screening in maternity wards with nurses and midwives as frontline implementers. Our study is a “real world” confirmation that the SickleSCAN device produces reliable results; that nurses are uniquely positioned to educate and advocate for newborn screening, and serves as a “proof of concept” for service delivery, particularly in settings where access is limited and point-of-care testing is less well studied. The authors do not declare any conflict of interest
P-093: PREVALENCE, KNOWLEDGE AND CLINICAL FEATURES OF PRIAPISM IN SICKLE CELL DISEASE IN SENEGAL
Authors: SECK M., GAYE O., TOURÉ S., GUÈYE S., KEITA M., DIALLO A., BOUSSO E., FAYE B., DIOP S.
Journal: HemaSphere (2022)

Purpose: The frequency of priapism is very high in adult sickle cell patients. In Africa, the assessment of the level of knowledge of priapism in sickle cell patients showed that priapism was poorly known. The objective of this study was to assess the knowledge, prevalence and clinical features of priapism in adult sickle cell patients in Senegal. Materials and methods: This was a seven-month descriptive study of 219 male patients with sickle cell disease (SS, SC, S-beta-thalassemia). SS sickle cell patients were in the majority. A questionnaire was drawn up with a 20-minute interview with each patient to assess his or her knowledge and the clinical features of priapism. Results: The mean age of the patients was 27.1 years (18-54) (SD=7.1). Twenty-three patients (10.5%) were aware of priapism (p=0.004). The prevalence of priapism in sickle cell patients was 41.5%. SS sickle cell patients were at higher risk (91.2%). Eighty-five patients (93.4%) had at least one episode of intermittent priapism and 24 patients (26.4%) had acute priapism. The mean age of onset of the first episode of priapism was 20.4 years +/- 6.1 years. The majority of patients (62.5%) had consulted within the first 24 hours. The last episode of priapism in 50.5% of patients was more than 6 months ago. Priapism occurred at night during sleep in 92.3% of patients. Fifty-eight patients (63.7%) did not have any factors favouring the occurrence of priapism. Conclusion: Priapism is a frequent serious vaso-occlusive complication in sickle cell disease. We show in this study, a high prevalence, the lack of knowledge of priapism and the clinical particularities of priapism in sickle cell disease in Senegal. The authors do not declare any conflict of interest
P-046: FACTORS ASSOCIATED WITH LEG ULCERS IN CAMEROONIAN SICKLE CELL DISEASE PATIENTS
Authors: EPOSSE C., NGOUADJEU E., PENDA C., NDEMBA CYNTHIA G., MBONO BETOKO R., EPEE EBOUMBOU P., MANDENG MA LINWA E., HASSANATOU I., LUMA NAMME H.
Journal: HemaSphere (2022)

Purpose: Leg ulcers are the most common skin complication in sickle cell patients. Though it is associated with great morbidity and cost of care, research is rather sparse on this condition. Through this study, we wished to determine the factors associated with leg ulcers in sickle cell patients in two hospitals in Douala. Materials and methods: This was a retrospective cross-sectional study carried out over a period of 05 years (January 01, 2015 to December 31, 2019). This study was carried out at two sites: the outpatient hematology department of the General Hospital Douala and Sickle cell care center at the Laquintinie hospital Douala. We included all sickle cell patients aged 10 years and above. We excluded patients with incomplete files. Data on sociodemographic characteristics and clinical parameters were collected. Analysis was done using SPSS version 25.0 software. Qualitative variables were compared using Pearson’s chi-square test. Multivariate analysis was done using logistic regression to determine the factors associated with leg ulcers. Significance level was set at p <0.05. Results: Out of 620 sickle cell patients, 41 (6.6%) had a leg ulcer. The median age of patients with a leg ulcer was 28 years (IQR 22-33). The male to female sex ratio was 1.15. Leg ulcers were traumatic in 70.7% of cases. The leg ulcer was unique in 65.85% (n=27) and located next to the malleolus in 26 (63.34%) of patients. Sociodemographic characteristics significantly associated with leg ulcers were the age groups 30-39 years [OR=9.3 (3.1-27.3), p <0.001], 20-29 years [6.3 (2.3-17.1), p <0.001] and a history of leg ulcer [2.7 (1.1-6.4), p=0.020]. For clinical characteristics, pulmonary hypertension [3.5 (1.7-6.7), p<0.001] and aseptic osteonecrosis of the femoral head [1.5 (0.01-2.25), p=0.020] were significantly associated with leg ulcers. Conclusion: In the pathogenesis of leg ulcer, a triad of mechanical obstruction of the microcirculation, hémolysis-vascular dysfunction syndrome and venous incompetence has been incriminated. in our study, one out of fifteen patients with sickle cell disease in Douala has a leg ulcer. Factors associated with leg ulcers were age between 20-39 years, pulmonary hypertension, aseptic osteonecrosis of the femoral head and history of leg ulcer. These factors are probably the result of chronic hyperhemolysis in our population. The authors do not declare any conflict of interest
P-094: EXPLORING SCHOOL LIVED EXPERIENCES OF SECONDARY STUDENTS WITH SICKLE CELL DISEASE THROUGH THEIR PARENTS’ LENS
Authors: HARDEN C.
Journal: HemaSphere (2022)

Purpose: The purpose of this qualitative study was to explore the academic and emotion of secondary students with sickle cell disease school experiences as their parents recalled them. Educators are responsible for ensuring access to quality education for all students. Students with disabilities have a legal right to a free appropriate public education under Section 504 and the Individualized Education Program (IEP). The chronic illness this research will focus on is children living with sickle cell disease. Sickle cell disease is an inherited blood disorder that causes severe pain episodes when sickled blood cells get stuck in blood vessels. To explore these experiences, the researcher employed a qualitative phenomenological approach to inquiry. The researcher also employed a social constructivism approach. Materials and methods: The study purpose was to investigate students’ academic and emotional school lived experiences through interviews and a focus group. The research method involved using the data from interviews, artifacts, and the focus group to analyze the parents’ experiences, background stories, and perspectives. The research method also included reviewing some parents’ artifacts, such as attendance records, grades, IEPs and 504 plans, and psychological evaluations to understand their educational attainment, absenteeism, and achievement. One participant shared the school attendance policy with the researcher as an additional artifact to provide additional insight into the phenomenon. Results: . The four themes that emerged were: (a) the need for a consistent approach by school staff, with awareness and understanding in an inclusive setting, (b) challenges faced by children with sickle cell disease in a school setting, (c) parents need for empowerment, and (d) need for adaptability in school culture and practices. The findings, results, and interpretations from this qualitative, phenomenological study support the need for increased awareness and support for secondary students with sickle cell disease as it relates to student achievement, emotion, and psychosocial challenges. Conclusion: Recommendation 1: Effective collaboration between schools and medical/psychosocial team Recommendation 2: Provide Targeted Professional Development Recommendation 3: Create Personalized Learning Options for Some Students with Sickle Cell Disease Recommendation 4: Promote Parent and Student Empowerment 1. Listen to mother’s story 2. Parent, Student and School Team Engagement Council 3. Parent Support Group 4. Review Hospital Homebound policy The authors do not declare any conflict of interest
P-018: MILESTONES ALONG MY PERSONAL JOURNEY WITH HYDROXYUREA
Authors: SHAKIR A.
Journal: HemaSphere (2022)

Purpose: For long sickle cell and thalassaemia were thought to be childhood diseases. It was believed that there was no future for these kids but to suffer the consequences of inheriting the genes and so their future was unplanned. I am Shifneez, 39 years old sickle beta-thalassaemic from Maldives. My parents were told I will not live up to be 14. I grew up as a very active student in school, graduated in teaching and worked at the very school I studied at. Materials and methods: I had my first sickle crisis at the age of 14. After about 5 years of not knowing what sickle cell can do, I was introduced to this drug called Hydroxyurea (HU) in 2003. After I got married and was ready to have a family, I was advised by my physicians to avoid becoming pregnant while taking HU. Understandably, they told me about the potential harm to the fetus. I was left with a real dilemma: Take a drug with unknown effects on fetal development or give up a helpful, necessary medication that has kept me healthy since I started it. Results: Many SCD patients are not willing to take a chance and go through pregnancy, let alone take the risk of continuing pregnancy with HU. hence, there is not a lot of clinical evidence with pregnancy while taking HU. My determination to start a family, despite being aware of the risks associated, past 17 years, I have had 3 pregnancies each experience different with regard to HU therapy. Today, I am a mother of 2. I had my last pregnancy and lactation while taking HU. Conclusion: HU, the only disease-modifying therapy approved for SCD, has continuously shown to improve the quality of life of adults and children with sickle cell. I hope the story of my personal journey can break the barrier and open the possibility for others like me to reach their milestones and dreams.Growth Chart of kids with and without exposure to Hydroxyurea during pregnancy and lactationExperience: ferritin level through Hydroxyurea therapy Vs Blood transfusion The authors do not declare any conflict of interest
P-075: HYDROXYUREA: EFFECTS ON TREATMENT OF CHILDREN WITH HEMOGLOBINOPATHY SC
Authors: CAMPOS A., PARK M.
Journal: HemaSphere (2022)

Purpose: Hemoglobin SC disease is the second most common genotype of sickle cell disease (SCD), and is characterized by erythrocyte dehydration, intra erythrocyte crystal formation and increased blood viscosity. Although it is considered less severe than SS and SB-0 genotypes, it may be associated to potential severe comorbidities, as avascular necrosis, retinopathy, vaso-occlusive crisis (VOC) and acute chest syndrome (ACS). These complications can cause low quality of life and justify surveillance and timely intervention. Evidence-based recommendations for its clinical management are lacking, with much of the treatment extrapolated from sickle cell anemia. This study describes the clinical profile of pediatric patients with hemoglobinopathy SC treated with hydroxyurea in a reference service in Brazil, outcomes and HU- related toxicity. Materials and methods: Retrospective single-center study that reviewed all children with hemoglobin SC disease treated with hydroxyurea in a tertiary hospital in Brazil, between 2009 and 2020, for at least 12 months by evaluating the laboratory profile, the number of episodes of VOC and ACS and the drug-related toxicity. Results: Eight patients have met the inclusion and exclusion criteria and were included in the study. There was a significant reduction in the number of episodes of vaso-occlusive crisis and acute chest syndrome after 12 months of treatment compared to the previous 12 months. Median age of start of HU treatment was 13 y. Six patients had neutropenia as medication toxicity, but only 2 of them failed to reach the maximally tolerated dose. One patient had transient thrombocytopenia. There were no other cases of significant toxicity. Conclusion: The use of hydroxyurea in the study group of children with hemoglobinopathy SC was shown to be safe and associated to a lower incidence of episodes of vaso-occlusive crisis and acute chest syndrome with minimal toxicity.Table 1. Clinical and demographic data of 8 children with hemoglobinopathy SC treated with HU between 2009 and 2020 in a tertiary hospital in BrazilTable 2. Comparison between laboratorial data before treatment, six months, and 12 months after treatment with HU in children with hemoglobinopathy SC The authors do not declare any conflict of interest
P-051: ENVIRONMENTAL DETERMINANTS IN MORBIDITY &amp; MORTALITY IN CHILDREN WITH SICKLE CELL DISEASE: A PROSPECTIVE OBSERVATIONAL STUDY AT A TERTIARY CARE CENTRE
Authors: SEBASTIAN R., JAIN D.
Journal: HemaSphere (2022)

Purpose: India has been identified as having the 2nd most number of births with sickle cell anemia in the world, after Africa. Apart from biomarkers, environmental factors play an important role in the clinical severity. As there are no such studies from India,current study attempts to address the effects of environmental factors in children with SCD. Materials and methods: Prospective observational study was conducted in 122 consecutively recruited hospitalized children of 1-12 years of age with SCD diagnosed by HPLC,living within a 10km radius of the tertiary care centre.Convenience sampling method was used.This a hospital based study conducted from 1st January 2020 to 31st December 2021.The severity of morbidity were identified by duration of hospital stay,number of blood transfusions,infections & VOCs and correlated with various environmental factors like temperature,rainfall,humidity,windspeed & particulate matters on the day of admission. Results: The study was conducted in Jan 2020-Dec 2021.A total of 212 admissions from 122 subjects were included, 88 females(41.51%) & 124 males(58.49%).Of this,35.85% of children were under 5 years & 64.15% of children above 5 years.The causes of admission included vasoocclusive crisis(54.25%),severe anemia requiring blood transfusions(41.98%) & infections(34.91%). Various environmental factors recorded during this period were as follows, maximum temp 32.85(22-46.8),min temp 21.46(0-30.8), relative humidity 78.41(20-100),wind speed 5.98(0-28km/hr),rainfall 4.89(0-100.3), atmospheric NO2 levels 27.29(0-95ug/m3),SO2 7.06(0-39ug/m3) & Ozone 41.76(0-148ug/m3). We noted an increase in the admissions as the relative humidity increases.Both extremes of temperature & relative humidity were the 3 important factors in causing VOCs & infections across 3 different seasons(p=0.0001).Among air pollutants, rising NO2 levels were significantly related to VOCs(p=0.0002).Rainfall had a strong correlation with infections(p=0.0035).All the environmental factors, except ozone were found significantly correlated with VOC & severe anemia while Infection was significantly related to all the environmental factors except wind speed,NO2 & Ozone. Conclusion: Studying the effect of environmental factors in SCD is important,as it allow patients and families to be given accurate information on how to reduce the risk of acute complications, because they can be manipulated relatively easy with simple advices,unlike genetic factors.However,further long term studies need to be conducted, to assess the effects on chronic complications and to drive public health policies. The authors do not declare any conflict of interest
P-065: IMPACT OF SOCIOECONOMIC FACTORS ON COGNITIVE FUNCTION IN CHILDREN AND ADOLESCENTS WITH SICKLE CELL DISEASE
Authors: DARBARI A., CONNOLLY M., HARDY S.
Journal: HemaSphere (2022)

Purpose: Sickle cell disease (SCD) is one of the most common hemoglobinopathy, affecting more than 300,000 infants each year globally and 1 in 365 Black or African American births in the United States. SCD is associated with various potential complications, including, but not limited to pain, acute chest syndrome, splenic sequestration, and neurological insults, among others. Patients with SCD who have a history of silent or overt cerebral infarcts or strokes are at risk for cognitive deficits. There is also evidence to suggest SCD carries risk for cognitive deficits even in the absence of silent or overt strokes, and factors associated with such deficits are not well understood. Sickle cell disease (SCD) is one of the most common hemoglobinopathy, affecting more than 300,000 infants each year globally and 1 in 365 Black or African American births in the United States. SCD is associated with various potential complications, including, but not limited to pain, acute chest syndrome, splenic sequestration, and neurological insults, among others. Patients with SCD who have a history of silent or overt cerebral infarcts or strokes are at risk for cognitive deficits. There is also evidence to suggest SCD carries risk for cognitive deficits even in the absence of silent or overt strokes, and factors associated with such deficits are not well understood. Materials and methods: The study was approved by Children’s National Hospital Institutional Review Board. Sixty-nine study participants (7-16 years old) completed the Wechsler Intelligence Test for Children Fifth Edition (WISC-V), where we collected Full Scale Intelligence Quotient (FSIQ), working memory, processing speed, and verbal comprehension. Socioeconomic factor data were collected from the Census and Maryland Department of Education, based on the participants’ postal code. Results: Overall, there were no significant correlations between socioeconomic factors and cognitive function (weak correlations ranging from r = 0.1 to r = 0.21, ps > .05). The highest correlation was 0.21 between processing speed and the number of libraries within a zip code, and this was not statistically significant (p > .05). Conclusion: These data suggest that community-level socioeconomic factors do not appear to relate to an individual’s cognitive functioning. That said, socioeconomic factors on an individual or familial basis may have an impact on cognitive function in children and adolescents with SCD, and further exploration is needed. Particular attention to cognitive functioning and literacy-based resources may help to elucidate the non-significant though larger correlation found in our data. Limitations of our study include small population size, and limited data on socioeconomic factors. In the future, a larger scale study with person-specific socioeconomic data rather than community level data could potentially describe relationships further. The authors do not declare any conflict of interest